The coexistence of significant congenital cardiac disease with accessory pathways is not uncommon, and presents a number of issues for clinical management. These issues include the propensity of such patients to have poorly tolerated tachycardia; the difficulties in choosing appropriate medical management given potential problems with preexisting sinus node disease, poor contractility, and the possibility of proarrhythmia; the need to seriously consider ablation prior to, or at the time of, intracardiac repair of congenital cardiac defects; and finally, the difficulties that complex anatomy may pose to the interventional electrophysiologist. Definitive management requires expertise both in electrophysiology and in pediatric cardiology. Success can usually be achieved with a careful, anatomically precise approach, combined with imagination and persistence. Because of the difficulties with medical management, catheter ablation may be the most attractive modality for control of accessory pathway tachycardia in this patient population.